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A rare, chronic and progressive encephalitis in children and adolescents, involving the white matter of the cerebrum, brain stem, cerebral cortex, thalamus, and spinal cord.

Description

A rare, chronic and progressive encephalitis in children and adolescents, involving the white matter of the cerebrum, brain stem, cerebral cortex, thalamus, and spinal cord. This is an almost invariably mortal disease caused by a chronic infection of measles with a defect reproduction of virus. It usually affects children younger than 12 years, but onset may be between the age of 5 and 20. There is a history of measles before 2 years of age.

After an asymptomatic period there is a gradual onset with intellectual deterioration, and jerky movements of trunk and extremities. The condition then progrediates fast with Babinski signs, bilateral spasticity, decerebrate rigidity, cachexia and dementia. Vision usually spared until last stage. Younger children die within months; adolescents may survive for months or even years.

The diseases picture was first described as a case report in 1939 by van Bogaert and Jacques de Busscher, both neuropathologists in Berchem-Anvers. Previous description in 1922 by the German neuropathologist Walther Spielmeyer (1879-1935), in 1931 by the German neurologists Gustav Bodechtel and E. Guttmann, in 1939 by Heinrich Pette (1887-1964) and G. Döring, both neurologists.

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